carnitine/seizures

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A newborn with seizures born to a mother diagnosed with primary carnitine deficiency.

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Maternofetal carnitine transport through the placenta is the main route of fetal carnitine uptake. Decreased free carnitine levels discovered by newborn screening has identified many asymptomatic adult women with systemic primary carnitine deficiency (PCD). Here, we presented amplitude

Suppression of pentylenetetrazol-induced seizures by carnitine in mice.

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When ddY mice were pretreated with L-carnitine (5, 10 or 20 mmol/kg), clonic as well as tonic seizures induced by pentylenetetrazol (PTZ) were dose-dependently suppressed. A time/response study (PTZ was injected 1, 5, 15 or 30 min after L-carnitine) showed that the anticonvulsive effects were

Dietary supplementation with acetyl-l-carnitine in seizure treatment of pentylenetetrazole kindled mice.

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In spite of the availability of new antiepileptic drugs a considerable number of epilepsy patients still have pharmacoresistant seizures, and thus there is a need for novel approaches. Acetyl-l-carnitine (ALCAR), which delivers acetyl units to mitochondria for acetyl-CoA production, has been shown

Early administration of vitamins B1 and B6 and l-carnitine prevents a second attack of acute encephalopathy with biphasic seizures and late reduced diffusion: A case control study.

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Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is the most prevalent encephalopathy in Japanese children. AESD is characterized by a prolonged febrile seizure on day 1 followed by secondary seizures and MRI abnormality on days 4-6, resulting in high

l-Carnitine Modulates Epileptic Seizures in Pentylenetetrazole-Kindled Rats via Suppression of Apoptosis and Autophagy and Upregulation of Hsp70.

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l-Carnitine is a unique nutritional supplement for athletes that has been recently studied as a potential treatment for certain neuropsychiatric disorders. However, its efficacy in seizure control has not been investigated. Sprague Dawley rats were randomly assigned to receive either saline (Sal)

CSF levels of carnitine in children with meningitis, neurologic disorders, acute gastroenteritis, and seizure.

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Carnitine concentrations in CSF, serum, and urine in normal febrile children and children with meningitis, neurologic disorders, and dehydration were studied. Carnitine levels in CSF were 1/10 compared with serum in normal febrile children. These levels increased two- to three-fold in the pathologic

Primary carnitine deficiency presenting as intractable seizures.

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The isolation of gamma-butyrobetaine, crotonbetaine and carnitine from brains of animals killed during induced convulsions.

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Neonatal carnitine palmitoyltransferase II deficiency associated with Dandy-Walker syndrome and sudden death.

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Neonatal onset of carnitine palmitoyltransferase II (CPT II) deficiency is an autosomal recessive, often lethal disorder of the mitochondrial beta-oxidation of long-chain fatty acids. It is a rare multiorgan disease which includes hypoketotic hypoglycemia, severe hepatomuscular symptoms, cardiac

Fatal carnitine palmitoyltransferase II deficiency in a newborn: new phenotypic features.

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We describe the term male infant of asymptomatic, healthy nonconsanguineous parents presenting on the first day of life with nonketotic hypoglycemia, seizures, hepatomegaly, cardiomegaly with biventricular hypertrophy, and ventricular arrhythmias. Cranial ultrasound revealed cystic dysplasia with

Carnitine conjugate of nipecotic acid: a new example of dual prodrug.

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As a novel example of improved entry of poorly delivered drugs into the brain by means of nutrient conjugates, L-carnitine was conjugated to nipecotic acid and the capacity to antagonize PTZ-induced convulsions of this novel entity was evaluated.

Carnitine-acylcarnitine translocase deficiency.

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Carnitine-acylcarnitine translocase deficiency, like other defects of mitochondrial fatty acid oxidation, is an autosomal, recessively inherited disorder. When the deficiency is near total, it is usually fatal, affects life soon after birth, and constitutes one of the causes of skeletal muscle

Neonatal Carnitine Palmitoyltransferase II Deficiency: A Lethal Entity.

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Carnitine palmitoyltransferase II (CPTII) deficiency is a rare disorder of mitochondrial fatty acid oxidation with autosomal recessive mode of inheritance. Three classic forms of CPT II deficiency have been described namely the lethal neonatal form, severe infantile hepatocardiomuscular form and the

Protective synergic effect of coenzyme Q10 and carnitine on hyperbaric oxygen toxicity.

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The comparative biochemical activities of coenzyme Q10 and carnitine can explain the protective synergistic effect of combination of these two substances in preventing the hyperbaric oxygen toxicity in mice. Both convulsions and mortality percentages are more significantly reduced in treated animals

Detection of neonatal carnitine palmitoyltransferase II deficiency by expanded newborn screening with tandem mass spectrometry.

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The introduction of tandem mass spectrometry to newborn screening has substantially expanded our ability to diagnose metabolic diseases in the newborn period. We report the first case of neonatal carnitine palmitoyltransferase deficiency II detected by expanded newborn screening with tandem mass

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